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Anemia

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Anemia

  1. 1. Anemia Prepared by: • Shkar Yasin • Rawezh Khasraw • Shkar Fayaq • Gashtyar Baxtyar
  2. 2. Outline 1. Definition 2. Normal RBC destruction 3. Classification 4. Hemolytic anemia 5. Diagnosis and treatment
  3. 3. Haematopoiesis • Blood cellular components formation derived from haematopoietic stem cells.
  4. 4. “WITHOUT BLOOD” • Decreasing number of RBC , haemoglobin or haematocrit below normal range for age and sex • Normal HB range between (13.5 – 17.5) for men, (12-15.5) for women • Infants have higher HB level
  5. 5. Normal red cell distruction
  6. 6. Classification:
  7. 7. Tests show increased RBC destructrion • Serum unconjugated bilirubin • Urobilinogen in urine • Stercobilinogen in feces • Raised LDH level • Serum haptoglobin • Hemoglobinuria, hemoglobinemia, hemosedrinuria in intravascular hemolysis.
  8. 8. Intravascular haemolysis
  9. 9. Intravascular haemolysis 1. Mismatched blood transfusion. 2. G6PD with oxidant stress 3. Red cell fragmentation syndrome 4. Cold autoimmune haemolytic anemia 5. Paroxysmal nocturnal haemoglobinuria
  10. 10. Hereditory Spherocytosis • Abnormality of RBC’s membrane • AD • Jaundice (extravascular haemolysis) • Spleenomegaly • Reticulocytosis(5-20%0
  11. 11. Investigation 1. Osmotic fragility 2. Negative direct antiglobulin 3. CBC
  12. 12. Hereditary Elliptocytosis • AD, defect in cytoskeletal membrane • 90% not anemic ,10% have same clinical manifestation as Spherocytosis
  13. 13. Treatment of spherocytosis and elliptocytosis 1. Surgery 2. Vitamins 3. Blood transfusion
  14. 14. G6PD Deficiency Anemia
  15. 15. G6PD deficiency anemia • Due to point mutation or deletion of enzyme G6PD causing less activity of the enzyme than normal. • Oxidant stress examples: 1. Drugs 2. Infections 3. Favism
  16. 16. Diagnosis of G6PD • Blood test for G6PD enzyme level • Blood film during crises showing blister and bite cells
  17. 17. irregularly contracted cells and blister cells
  18. 18. Clinical manifestations • Asymptomatic between crises • During crises patient experience 1. Yellowish skin 2. Shortness of breath 3. dark urine 4. Haemoglobinemia 5. Haemosiderinuria
  19. 19. Thalassemia • Inherited blood disorder. • Thalassemia is an AR genetic disorder. • Inadequate Hb production
  20. 20. slideplayer.com ThalassemiaGerald A. Soff
  21. 21. Classifications • Beta thalassemia • Alpha thalassemia
  22. 22. Beta thalassemia Major • Severe form of beta thalassemia • Presence of two abnormal genes • Caused by mutation of HBB gene on chromosome 11
  23. 23. pathophysiology
  24. 24. Clinical manifestation: 1. anemia 2. Iron overload 3. Pallor 4. Jaundice 5. Skull deformity, mongoloid face 6. Cardiac dysfunction or failure
  25. 25. Blood picture 1. Hypochromic microcytic anemia 2. Decrease MCV, MCH 3. HB electrophoresis.
  26. 26. Treatment: symptomatic relieve 1. Blood tranfusion 2. Iron chelating drugs like Deferoxamine 3. Speleenectomy 4. Bone marrow transplantation
  27. 27. Betathalassemia minor • Presence of one abnormal gene • Slight reduced Hb level • RBC count. • Blood film shows anisocytosis, hypochromia, target and tear cells
  28. 28. Diagnosis of beta thalassemia minor • Increased HbA2 4 to 7 percent • TIBC is normal
  29. 29. Clinical features+ diagnosis of HBH disease • Microcytic hypochromic anemia • Spleenomegaly • Mild jaundice • Pallor
  30. 30. Golf ball appearance
  31. 31. Prevention • Premarital screening • Prenatal diagnosis • Pre implantation genetic diagnosis
  32. 32. Sickle cell anemia • Inherited Structural abnormality • Substitution of glutamic acid by valine
  33. 33. Clinical manifestation • Episodes of pain (crisis) • Painful swelling of hand and fit • Frequent infection • Delayed growth due to lowering O2 • Vision problems
  34. 34. Complications 1. Stroke 2. Acute chest syndrome 3. Pulmonary hypertension 4. Blindness 5. Leg ulcer
  35. 35. Investigation and diagnosis • Blood film: normochromic, microcytic, • retoculocytosis 10-20% • Sickling test • HbS 80-95% in severe cases
  36. 36. Iron deficiency anemia • Iron deficiency is cause defect in hemoglobin synthesis , result in the RBC is smaller than normal (microcytic , hypochromic)
  37. 37. Cause of iron deficiency anemia 1. Inadequate iron intake 2. Mal absorption of iron 3. Increase iron requirement 4. Increase iron loss 5. Chronic blood loss 6. Subtotal or complete gastrectomy
  38. 38. Sings and syntom A. General fatigue B. weakness C. Pale skin D. shortness of breath E. dizziness F. tongue swelling G. cold hands and feet H. tachycardia I. brittle nails J. headaches
  39. 39. Diagnosis of iron deficiency anemia Blood film Microcytic and hypochromic CBC
  40. 40. treatment • Diet: meat , vegetable ,nuts • Iron supplement
  41. 41. What is megaloblastic anemia? • Hematological disorder • Deficency of folic acid and vit.B12
  42. 42. Vitamin B12 • Imprtant for synthesis DNA
  43. 43. Folic acid • Important in synthesis DNA
  44. 44. Cause of Megaloblastic anemia B12 deficiency Folic acid deficiency
  45. 45. B . Cause of vit.B12 deficiency Inadequate dietary intake Mal absorption 1.gastric cause 2.intestinal cause
  46. 46. A. Cause of folic acid deficiency Alcoholism Malnutrition Excessive cooking Drug: phenytoin, barbiturate Increase requirement : pregnancy ,lactation
  47. 47. Clinical manifestations: • Fatigue • Weakness • Body ache • Vertigo
  48. 48. • Beefy tongue • Diarrhea • Anorexia • Weight loss
  49. 49. Diagnosis? 1. Laboratory finding: A. Blood film: MCV increase Film: Normochromic, Anisocytosis poikilocytosis, macrocytosis, tear drop cells
  50. 50. 2. CBC A. RBC count reduce B. Heamoglobin reduce C. Mcv increase D. MCH increase E. MCHC normal or reduce
  51. 51. 3. Bone marrow finding: A. Hypercellular marrow. B. Erythropoisis, with megaloblastic maturation. C. Giant metamyelocytes.
  52. 52. 4.Biochemical finding: A. Vitamin B12 reduce B. Folate acid reduce C. Unconjugate bilirubin increase D. Serum iron and fertin may be normal or elevated
  53. 53. treatment • 1.treatment of B12 deficiency A. DIET
  54. 54. 2. medication: • 1000 ug B12 IM once a week for 8 weeks
  55. 55. 2.Treatment of folic acid deficiency • A. diet : vegetable, nuts, meat
  56. 56. B. medication • 5mg/day for 2 – 4 months
  57. 57. REFERENCE • Hoff brand essential heamatology 7th edition • Essential of clinical medicine (PROF.DR.MAGDY ISHAK) 2018 22edtion • DAVIDSON principle and practic of medicine( BRIAN.R WALKER)2018 22edition • DACIDSON ESSENTIAL OF MEDICINE (J.ALASTAIR INNES)2018 2 edition • First aid usmle 2018 • WWW.HEALTHCARE.COM • WWW.MYOCLINIC.COM • Google image

Notizen

  • In developing embryos, blood formation occurs in aggregates of blood cells in the yolk sac, called blood islands. As development progresses, blood formation occurs in the spleen, liver and lymph nodes. When bone marrow develops, it eventually assumes the task of forming most of the blood cells for the entire organism.[1] However, maturation, activation, and some proliferation of lymphoid cells occurs in the spleen, thymus, and lymph nodes. In children, haematopoiesis occurs in the marrow of the long bones such as the femur and tibia. In adults, it occurs mainly in the pelvis, cranium, vertebrae, and sternum.[8]
  • Macrophage phagocytes rbcs then destruct it to haem and globin, the globin part reused again, and haem changes to protoporphyrin and iron, iron release into circulation, and protoporphyrin change to bilirubin in liver, bilirubin relaeased to bile then released ethier by stool or urine
  • Only mention two or three examples of types, and in RBC low and high means RBC count with microcytic, if its high you must say its bcz compensatory action of bone marrow, bcz in thalassemia minor our BM try to produce more RBCs so lead to high RBC
  • First class we will discuss Haemolytic anemia

    Results from increasing rate of RBC destruction
    Life span of RBCs less than 3 days

    Intrinsic defect: hereditory
    hemoglobinopathies:like thalassemia ,sickle cell anemia
    Enzymopathies : like G6PD deficiency anemia
    Membrane cystoskeletal defect: spherocytosis,eleptocystosis

    Acquired
    Paroxysmal nocturnal hemoglobinuria: rare life threatening disease characterized by RBC destruction by complement system due to defective presence of a protein called DAF on the RBC(complement decay acclererating factor) its function is to inhibit complement system from RBC decstruction

    Extrinsic defect: hereditary
    Familial uremic hemolytic syndrome: rare renal failure, anemia, thrombocytopenia, is two types typical whichs due to ingestion of specific strain of bacteria release shi
    Ga toxin and cause enterohemorrhage means in intestine, atypical due to genetic mutation
    Acquired:
    Mechanical destructiuon like red cell fragmentation syndrome: The red cell fragmentation syndrome can occur due the blood vessels or vascular malformations
    Toxin agent domestic chemical substances
    Drugs associated
    Infection b y malaria
    Autoimmune warm and cold

  • Reduced serum haptoglobin(is a protein that binds to free haemoglobin released from erythrocyte ,hemoglobin haptoglobin complex removed by spleen, bcz in
  • Defect in protein due to gene mutation involved in interaction between the membrane cystoskeleton and the lipid bilayer of the RBC (Vertically), the cell membrane of RBC become fragile and unstable at early life’s RBC they are Biconcave but when they circulate become spherocyte,the cell membrane produce small fragment and in the macrophage the small fragements deattached and released as SPherocytes
  • The RBC become more hypertonic
    Means it’s not autoimmune haemolytic anemia
    Normochromic microcytic
  • There is no cure for HS, but it can be treated. The severity of your symptoms will determine which course of treatment you receive. Options include:
    Surgery: In moderate or severe disease, removing the spleen can prevent common complications that result from hereditary spherocytosis. Your red blood cells will still have their spherical shape, but they’ll live longer. Removing the spleen can also prevent gallstones.
    Not everyone with this condition needs to have their spleen removed. Some mild cases can be treated without surgery. Your doctor might think less invasive measures are better suited for you. For example, surgery is not recommended for children who are younger than 5 years.
    Vitamins: Folic acid, a B vitamin, is usually recommended for everyone with HS. It helps you make new red blood cells. A daily dose of oral folic acid is the main treatment option for young children and people with mild cases of HS.
    Transfusion: You may need red blood cell transfusions if you have severe anemia.
  • Before we discuss this types, we mention that RBC have a pathway called hexose monophosphate pathway, in this pathway generate NADPH whichs important for maintain reduced glutathione,which is important to prevent damage from free radicals
  • Any response to oxidant stress leading to intravascular haemolysis,because G6PD is not worked properly
    Drugs like premaquine sulphonamied,nitrofurantoins
    Infections like viral hepatitis , pneumonia
    Favism : ingestion of fava beans
  • Yellowish skin due to execcive RBC destruction
    Dark urine due to intravascular haemolysis
  • Commonly found in ethnic groups, whose origins are near the medesterian sea and equatorial or near equatorial region of Asia;middle east
    Commonest group of AR in India
    Carier frequencies vary from 3% to 17% in different populations,with over 30 million people carring the defective gene
    About more than 9000 thalasemic children are born every year
  • We classified like that bcz normal hemoglobin molecule contains 2 beta and 2 alpha chain.
    Defect in each chain lead to thalassemia


    Beta: characterized by genetic deficiency in the synbthesis of beta globin chain

    Alpha: change in the genes for alpha globin production
  • Present before age of 2 years
    Transfusion dependent
  • When theres excessive decrease in the beta globin change leading to decrease adult haemoglobin and as compensatory mechanism the body produce Haemopoisis in the spleen and liver which produce fetal haemoglobin ,then leading to improper delivery of oxygen to the tissue and hepatospleenomegaly,in the other hand the exess alpha chain in RBC precursor produce defect in RBC metabolism and membrane leading to destroying RBC in the extravascular organ like bone marrow
  • Iron overload : Due to excessive rbc destruction
    Jaundice due to excessive unconjugated bilirubin
    Skull deformity due to erythroid hyperplasia and intramedullary expansion and cortical bone thining
    Heart failure due to iron overload and deposition in epicardium
  • To prevent iron overload and decrease risk of heart failure we use iron chelating drugs like deferoxamine


    Spelenectomy to decrease RBC destruction and decrease transfusion requirement, and to prevent complications caused by splenomegaly
  • Why RBC above normal bcz bone marrow try compensate of abnormal Hb
  • Generally develops in first years of life , intial signs may not be noticed until during routingly haematological analysis
    Survive adult to life
  • Electrophresis: showing HbA with 5-40% HbH

    HbH appear in blood film as golf ball like appearance
  • Premaretal screening to identify coules at risk of bearing affected children, by estimating (looking HbA2 and HbF level, prenatal screening allows the couples the choice of termination
    Tests one or two cells from each embryo for a specific genetic abnormality , usese for IVF embryos
     form of assisted reproductive technology that combines preimplantation genetic diagnosis with in vitro fertilization may help parents who have thalassemia or who are carriers of a defective hemoglobin gene give birth to healthy babies
  • Is a type of anemia results from genetic change of Hb due to exchange of Glutamic acid by valine amino acid
    RBCs become rigid and sticky and sickle shape
    RBCs is insoluble and form crystal when expose to low oxygen
    There are not enough healthy RBC to carry adequate oxygen
  • Major symptoms of sickle cells anemia:
    Pain develop when sickle block blood flow through tiny blood vessel to ur chest, abdomen and joints
    Painful swelling due to blocking blood flow
    Sickle cell can damage organs that fight against infection
  • Acute chest syndrome characterized by fever, and difficulty in breathing, caused by lung infection
    Blindness due to chronic blood vessel obstruction
  • Sickling test :
  • Iron deficiency anemia is the most common type of anemia, and it occurs when your body doesn’t have enough of the mineral iron. Your body needs iron to make hemoglobin. When there isn’t enough iron in your blood stream, the rest of your body can’t get the amount of oxygen it needs.
    Daily requir ment is 1 mg for male
    And 2 mg for female
  • 2. Malabsorbtion due to hypochloryhydria and diarrhea
    3. As in childeren and pregnancy
    4 as injury
    5 menstral and menoposal bleeding
  • VITAMIN B12 IS IMPORTANT FOR SYSNTHESIS DNA AND DEFICIENCY OF VIT B12 LEAD TO DELAY NUCLEAR MATURATION AND CELL DIVISION .
    VIT B12 SYNTHESIS IN HUMAN LARGE BAWEL BUT CAN NOT ABSORBED , HUMAN DEPEND ON DIETARY SORCE . AFTER ABSORBED FROM LOWER ILIUM STORE IN LIVER . THE STORED B12 MAY LAST FOR 3 YEARS DAILY REQUIRMENT IS 1 -2 MG PER DAY
  • FOLIC ACID IS Is imprtant for sysnthesis dna and rna and to repair and aiding in rapid cell devision and growth and to produce healthy rbc
    Folic acid is absorbed in jejenum daily requement is 400 mg per day
    Folic acid is stored in liver
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